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  • 1
    ISSN: 1432-1440
    Keywords: Pseudohermaphroditism ; Gynecomastia ; Androstenedione ; 17β-Hydroxysteroid-oxydoreductase deficiency ; Testosterone inborn error ; Pseudohermaphroditismus masculinus ; Gynäkomastie ; Androstendion ; 17β-Hydroxysteroid-oxydoreductase Defekt ; Testosteron-Synthesestörung
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Beschreibung eines Jungen mit Pseudohermaphroditismus maskulinus, mit normalem männlichen Karyotyp, welcher bei der Geburt als Mädchen angesehen worden war. Mit 12 Jahren Auftreten einer hochgradigen Gynäkomastie. Steroiduntersuchungen wurden mit Radiogaschromatographie und mit radioimmunologischen Methoden durchgeführt. Die Steroidbestimmungen ergaben im Plasma bei altersnormalen Testosteronspiegeln Androstendionwerte, welche die Norm um den Faktor 9–23 überstiegen. Die Plasmaoestrogene wurden insgesamt erhöht gefunden, Oestron weit mehr als Oestradiol. In Inkubationsversuchen vom bioptisch gewonnenen Hodengewebe des Jungen konnte gezeigt werden, daß zugesetztes markiertes Androstendion nur in geringem Maß zu Testosteron metabolisiert wurde, während normales Hodengewebe rund 60% zu Testosteron metabolisierte. Auf Grund dieser biochemischen Befunde wird eine Testosteron-Synthesestörung mit Defekt einer 17β-Hydroxy-steroid-Oxydoreduktase als Ursache des Pseudohermaphroditismus und der Gynäkomastie angenommen. Der Enzymdefekt ist bisher zweimal beschrieben, scheint aber nach entsprechender Untersuchung häufiger zu sein.
    Notes: Summary A boy with male pseuodhermaphroditism described here with normal male caryotype was at first raised as a girl. The error was detected within the first year of life. At the age of 12 years the boy developed a severe gynecomastia. At this time determinations of plasma and urinary steroids were undertaken. 17-ketosteroids and total 17-OHCS were determined by colorimetry. Plasma testosterone, androstenedione, 17α OH-progesterone, urinary pregnantriol and pregnantriolone were measured by GLC. Plasma estrone and estradiol were determined by radioimmunoassay (RIA). Investigations at 12 years of age and later revealed plasma testosterone in the lower normal range and androstenedione 9–23 times higher compared to normal boys. Plasma estrogens were found to be elevated, estrone much more than estradiol. Incubation studies with testicular tissue showed very little conversion of3H-androstenedione (15%) whereas normal testicular tissue converted up to 60% to3H-testosterone. On the basis of these data 17β-hydroxysteroid-oxydoreductase deficiency is suggested as the underlying cause of pseudohermaphroditism and gynecomastia in this boy. Up to the present this enzyme deficiency is described only two times but it would seem to be more frequent if one looked at the elevated androstenedione plasma level.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Journal of molecular medicine 62 (1984), S. 1011-1013 
    ISSN: 1432-1440
    Keywords: Etomidate ; Adrenocortical suppression ; 17-Deoxysteroids ; 17-Hydroxysteroids
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary To investigate the adrenocortical suppression caused by the anesthetic etomidate, plasma levels of progesterone (P), 17-hydroxyprogesterone (17-OHP), 11-deoxycorticosterone (DOC), corticosterone (B), aldosterone (Aldo), 11-deoxycortisol (S), cortisol (F), and cortisone (E) were measured simultaneously before and after a short-term ACTH stimulation test in a 6.5-year-old boy whose convulsions could be kept under control only with constant etomidate infusions. During etomidate therapy, plasma levels of DOC and S were extremely elevated, the progestins P and 17-OHP were slightly elevated, whereas B and Aldo were in the lower normal range, and F and E were markedly decreased. A short-term ACTH stimulation test during etomidate infusion gave a blunted response of B, Aldo, F and E, whereas the level of DOC remained high and S even further increased. P and 17-OHP showed a positive response to ACTH. The ratios of B/DOC and F/S, which reflect adrenocortical 11β-hydroxylase activity, were extremely decreased during etomidate and did not change after ACTH stimulation. In contrast, the ratios of DOC/P and S/17-OHP, which relect 21-hydroxylase activity, were elevated and remained elevated after ACTH stimulation. After discontinuation of etomidate therapy, all the baseline steroid levels were somewhat elevated, but responded normally to ACTH. These results demonstrate that etomidate causes a specific and reversible blockade of the 11β-hydroxylation of adrenal steroid synthesis.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-1440
    Keywords: Atrial natriuretic peptide ; Cyclic GMP ; Volume loading
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary To investigate the effects of fluid expansion on endogenous atrial natriuretic peptide (ANP) and cyclic 3′,5′-guanosine monophosphate (cGMP), four male volunteers were studied before, during and after intravasal volume loading. Volume expansion was performed by intravenous infusion of 2,000 ml isotonic saline solution within 30 min. Mean plasma ANP levels increased 2.5-fold from 31.2 pg/ml to 81.7 pg/ml 40 min after the start of infusion. Plasma cGMP levels paralleled the rise in ANP, shwoing a mean cGMP increment from 2.7 pmol/ml to a maximum of 8.2 pmol/ml. Both ANP and cGMP levels were back to basal levels 120 min after termination of the infusion. Stimulation of endogenous ANP release by volume loading suggests that ANP is involved in the regulation of fluid homeostasis in man. The parallel rise in plasma cGMP levels supports the idea that cGMP is a mediator for the effects of ANP.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-1440
    Keywords: Osteogenic sarcoma ; Methotrexate ; Radioimmunoassay ; Osteosarkom ; Methotrexat ; Radioimmunoassay
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Bei 22 Patienten mit osteogenem Sarkom, die 103 hochdosierte Methotrexatinfusionen (6–8,5 g/m2 in 4–6 h) erhielten, wurde mit einem eigenen spezifischen und rasch durchführbaren Radioimmunoassay die Plasmapharmakokinetik des Methotrexats untersucht. Bei nichttoxischen Verläufen lag die Plasmakonzentration nach 24 h unter 8,0 × 10−6 mol/l, nach 48 h unter 8,0 × 10−7 mol/l und nach 72 h unter 4,25 × 10−7 mol/l. Alle Patienten mit 48 h-Werten über 1 × 10−6 mol/l entwickelten schwere toxische Erscheinungen in Form von Knochenmarksdepression und Stomatitis, die durch eine verzögerte Ausscheidung des Methotrexats bedingt war. Der Anstieg des Serumkreatinins war kein zuverlässiges Kriterium für toxische Verläufe. Die Bestimmung der 48- und 72h-Methotrexat-Plasmakonzentrationen erwies sich als zuverlässiger Parameter zur Erfassung von Patienten mit drohender Toxizität. Sie ermöglicht somit, rechtzeitige therapeutische Maßnahmen, z.B. in Form einer zusätzlichen Leukovorintherapie zu ergreifen.
    Notes: Summary In 22 patients with osteogenic sarcoma, treated with 103 high-dose methotrexate infusions (6–8.5 g/m2 in 4–6 h) plasma methotrexate levels were measured with a specific and rapid radioimmunoassay. Nontoxic infusions were associated with methotrexate concentrations below 8.0 × 10−6 mol/l at 24 h, 8.0 × 10−7 mol/l at 48 h and 4.25 × 10−7 mol/l at 72 h. All patients with 48 h methotrexate levels above 1 × 10−6 mol/l manifested severe toxicity with myelosuppression and stomatitis due to delayed methotrexate excretion. Rise of serum creatinine was not reliable to predict oxicity. Determination of 48-and 72-h methotrexate concentrations proved to be a valuable method for identifying patients at high risk for toxic side effects. Additional citrovorum factor may thus be given in time.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-1440
    Keywords: Coronary heart disease ; Cardiopulmonary bypass surgery ; Postoperative care ; Prethrombotic state ; Mean platelet volume ; Platelet distribution width ; Platelet count ; Leukocyte count
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We studied the effect of coronary-artery bypass surgery on blood cells and platelets. Hematological parameters of eighty-three patients were measured by an automated cell counting and sizing analyzer. Sampling time was from 24 h prior to 10 days after surgery. During this time leukocytes and platelets showed characteristic changes in numbers and size, whereas red blood cells revealed no typical modifications. Even though it seems clear that changes of hematological parameters occur after bypass surgery, it is important to be aware of the actual extent of such changes. Therefore the data of 50 patients who had had no post-operative clinical complications were combined to generate diagrams of those parameters that had changed in a characteristic fashion. The diagrams showing average changes, and 99% confidence intervals in mean platelet volume and platelet count were able to identify seven (out of 7) cases with complications up to 48 h before clinical signs were apparent. Complications ranged from mild (3 cases with infections) to severe (4 cases with thrombosis, embolic thrombosis and/or reinfarction). Diagrams showing changes in leukocyte parameters were able to identify only two cases with infections. Even though the number of cases is yet small, the results suggest that surveillance of platelet parameters may be useful in postoperative care. Furthermore, this study was able to confirm the recent findings of Trowbridge and Martin [18] that an abnormal increase in platelet volume distribution width and low platelet counts found in patients with coronary heart disease may serve as good indicators for the prethrombotic state and the risk of myocardial infarction.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-1076
    Keywords: 17-OH-Progesterone ; Androstenedione ; Testosterone ; Normal values ; Prepubertal patients with congenital adrenal hyperplasia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract To determine maximal plasma levels of androstenedione (A) and testosterone (T) which still can be considered non virilizing in 21-hydroxylase deficiency, we investigated plasma levels of these steroids in unaffected children and in adults. For T we found an upper limit of the prepubertal normal range of 16 ng/dl in girls and of 20 ng/dl in boys, with the exception of boys in the first half-year of life in which T is elevated up to the low adult range with peak values in the 2nd and 3rd month. During puberty T values show a significant difference between pubic hair stage 1 and stage 2. T levels below 20 ng/dl can be considered to be non virilizing. For A we found a plasma concentration of 86 ng/dl to be the upper normal level in both sexes before the onset of puberty. A values below this limit are expected to be non virilizing. To evaluate the usefulness of 17-hydroxyprogesterone (OHP) for the prediction of A and T and T and to define “acceptable” OHP levels in CAH we performed simultaneous determinations of OHP, T, and A in prepubertal patients treated for CAH. From these values we calculated the 95% confidence interval for prediction of T and A on known OHP levels. On an OHP value of 1.000 ng/dl, T can be excpected to be between 6 and 60 ng/dl and A between 25 and 320 ng/dl. Because of these wide ranges, OHP has to be considered an unreliable parameter for predicting androgen levels in CAH.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1432-1076
    Keywords: Delayed puberty ; Growth rate ; Growth hormone concentration (mean daily growth hormone concentration) ; Testosterone concentration (mean daily testosterone concentration)
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The mean growth hormone concentration during a 24-hour period in 7 boys of short familial stature and a growth rate of 3.2–5.4 cm/year was between 1.0 and 4.6 ng/ml serum. In 7 boys with pubertal growth spurt and familial tallness (growth rate 7.2–11.0 cm/year) it varied from 0.97 to 4.4 ng/ml and in 6 boys with constitutional delay of puberty (a growth rate of 4.2–5.2 cm/year prior to puberty) from 1.3 to 4.3 ng/ml. No correlation was found between the 24-hour mean growth hormone concentration and the mean 24-hour testosterone concentration in serum or the growth rate, but a correlation was found between testosterone and the growth rate. It is concluded that the growth spurt in puberty is not due to a change in growth hormone concentration but rather to the increase of androgen production in puberty.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    European journal of clinical pharmacology 18 (1980), S. 95-104 
    ISSN: 1432-1041
    Keywords: corticosteroids ; progestins ; betamethasone ; phenobarbital ; amniotic fluid ; fetoplacental unit
    Source: Springer Online Journal Archives 1860-2000
    Topics: Chemistry and Pharmacology , Medicine
    Notes: Summary Corticosteroids (CS) are known to be essential for fetal organ maturation and seem to play an important role in both the initiation of parturition and the postnatal adaptation of the human neonate. Pharmacologically, CS are widely used for enhancing fetal lung maturation prior to premature delivery. However, knowledge of endogenous CS and precursor levels throughout fetal and perinatal life and their response to exogenous CS is limited. Therefore, using automated liquid column chromatography plus specific radioimmunoassays, unconjugated aldosterone (Aldo), corticosterone (B), 11-deoxycorticosterone (DOC), progesterone (P), 17-hydroxyprogesterone (17-OHP), 11-deoxycortisol (S), cortisol (F) and cortisone (E) were simultaneously followed in 70 amniotic fluid (AF) control samples throughout pregnancy, and in cord and neonatal plasma longitudinally during the first week of life. From 14 to 38 weeks, AF levels of all measured steroids except E rose by 2 to 12-fold on the average (allP〈0.001) but declined at term. E increased until 31–35 weeks (P〈0.01), then remained almost constant until term. Cord levels of all steroids were substantially higher than those found in AF at term. While levels of the placentally derived steroids P, 17-OHP, DOC and E dropped sharply after birth by several orders of magnitude (P≪0.01) showing typical disappearance curves, the biologically most potent CS Aldo and F rose even further immediately after birth. Whereas Aldo levels declined from maxima about 100 times above normal adult levels at 6 h by almost 3-fold until day 7 (P〈0.01), F (and also B) fluctuated considerably resembling a damped oscillation and, by day 7, reached mean levels less than half of those seen in later childhood. After betamethasone treatment of the mother, neonatal levels of Aldo and F were suppressed to 24–69% of normal until day 9, whereas those of the other steroids (except E) returned to normal during the first hours of life. Phenobarbital (PB) therapy of the mother led to decreased steroid levels in maternal and umbilical venous plasma at term, while umbilical arterial CS levels, notably those of Aldo and F (P〈0.02), were increased when compared with untreated controls, indicating a stimulation of the most potent CS in the fetus after PB. The significance of the findings in view of fetoplacental function and fetal organ maturation is briefly discussed.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    Biochemical and Biophysical Research Communications 151 (1988), S. 997-1003 
    ISSN: 0006-291X
    Keywords: [abr] CSA; cyclosporin A ; [abr] DMSO; dimethylsulfoxid ; [abr] PK-C; protein kinase C ; [abr] PMA; phorbol 12-myristate 13-acetate ; [abr] PMSF; phenylmethylsulfonyl fluoride ; [abr] PS ; [abr] TCA; trichloroacetic acid ; [abr] phosphatidylserine
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Biology , Chemistry and Pharmacology , Physics
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    Biochemical and Biophysical Research Communications 197 (1993), S. 549-555 
    ISSN: 0006-291X
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Biology , Chemistry and Pharmacology , Physics
    Type of Medium: Electronic Resource
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